OBJECTIVES: To investigate the efficacy and feasibility of respiratory muscle training (RMT) in patients with spinal muscular atrophy (SMA) and respiratory muscle weakness. DESIGN: A 4-month, single-blinded RCT, followed by an 8-month extension phase. SETTING: The outpatient clinic of the Neurology department and the Child Development and Exercise Center at the University Medical Center Utrecht, The Netherlands. PARTICIPANTS: 30 participants, >= eight years, SMA type 1 to 4, and respiratory muscle weakness. INTERVENTIONS: The training group started RMT at 30% of their maximum inspiratory (PImax) and expiratory pressure (PEmax) and adjusted their training intensity based on perceived exertion. The control group started and continued RMT at 10% of PImax and PEmax. After four months, the control group received the same RMT as the training group. MAIN OUTCOME MEASURES: The primary outcome was the between-group difference in PImax after the RCT phase. Additionally, we explored the association between training volume (ie, number of training sessions x training intensity) and increase in PImax over 1 year. RESULTS: 25 participants completed the study. Mean PImax after four months (corrected for baseline value) training group: 61.1 cmH2O, control group: 54.3 cmH2O, mean difference in PImax: 6.8 cmH2O (95% CI -2.0 to 15.6). Despite the study being underpowered, we did find an association between training volume and increase in PImax (R2 0.531). CONCLUSION: There was a small between-group difference in PImax after the RCT phase. The training was safe, but the feasibility of the training needs further improvement. CLINICAL TRIAL REGISTRATION NUMBER: NL73280.041.20. CONTRIBUTION OF THE PAPER.

Full text (sometimes free) may be available at these link(s):      help

• DOI
• PubMed
• publisher